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Encyclopedia > Haemophilia
Hemophilia d
Classification and external resources
ICD-10 D66.-D68.
ICD-9 286
OMIM 306700 306900 264900
DiseasesDB 5555 5561 29376
MedlinePlus 000537
eMedicine med/3528 
MeSH D025861

Haemophilia (also spelled as hemophilia, from the Greek haima "blood" and philia "to love"[1]) is a group of hereditary genetic disorders that impair the body's ability to control blood clotting or coagulation. In its most common form, Hemophilia A, clotting factor VIII is absent. In Haemophilia B, factor IX is deficient. Hemophilia A occurs in about 1 in 5,000-10,000 male births[2], while Hemophilia B occurs at about 1 in about 20,000-34,000. The International Statistical Classification of Diseases and Related Health Problems (most commonly known by the abbreviation ICD) provides codes to classify diseases and a wide variety of signs, symptoms, abnormal findings, complaints, social circumstances and external causes of injury or disease. ... The International Statistical Classification of Diseases and Related Health Problems 10th Revision (ICD-10) is a coding of diseases and signs, symptoms, abnormal findings, complaints, social circumstances and external causes of injury or diseases, as classified by the World Health Organization (WHO). ... // C00-D48 - Neoplasms (C00-C14) Malignant neoplasms, lip, oral cavity and pharynx (C00) Malignant neoplasm of lip (C01) Malignant neoplasm of base of tongue (C02) Malignant neoplasm of other and unspecified parts of tongue (C03) Malignant neoplasm of gum (C04) Malignant neoplasm of floor of mouth (C05) Malignant neoplasm of... // C00-D48 - Neoplasms (C00-C14) Malignant neoplasms, lip, oral cavity and pharynx (C00) Malignant neoplasm of lip (C01) Malignant neoplasm of base of tongue (C02) Malignant neoplasm of other and unspecified parts of tongue (C03) Malignant neoplasm of gum (C04) Malignant neoplasm of floor of mouth (C05) Malignant neoplasm of... The International Statistical Classification of Diseases and Related Health Problems (most commonly known by the abbreviation ICD) provides codes to classify diseases and a wide variety of signs, symptoms, abnormal findings, complaints, social circumstances and external causes of injury or disease. ... The following is a list of codes for International Statistical Classification of Diseases and Related Health Problems. ... The Mendelian Inheritance in Man project is a database that catalogues all the known diseases with a genetic component, and - when possible - links them to the relevant genes in the human genome. ... The Disease Bold textDatabase is a free website that provides information about the relationships between medical conditions, symptoms, and medications. ... MedlinePlus (medlineplus. ... eMedicine is an online clinical medical knowledge base that was founded in 1996. ... Medical Subject Headings (MeSH) is a huge controlled vocabulary (or metadata system) for the purpose of indexing journal articles and books in the life sciences. ... A philia is the love or obsession with a particular thing or subject. ... The ancients had a variety of ideas about heredity: Theophrastus proposed that male flowers caused female flowers to ripen; Hippocrates speculated that seeds were produced by various body parts and transmitted to offspring at the time of conception, and Aristotle thought that male and female semen mixed at conception. ... A genetic disorder is a condition caused by abnormalities in genes or chromosomes. ... For other uses, see Blood (disambiguation). ... This article is about the clotting of blood. ... Haemophilia A (also spelt Hemophilia A or Hæmophilia A) is a blood clotting disorder caused by a mutation of the Factor VIII gene, leading to a deficiency in Factor VIII. It is the most common hemophilia. ... Factor VIII (FVIII) is an essential clotting factor. ... Haemophilia B (also spelled Grus, Hemophilia B or Hæmophilia B) is a blood clotting disorder caused by a mutation of the Factor IX gene. ... Factor IX (or Christmas factor or Christmas-Eve factor) is one of the serine proteases (EC 3. ...


The effects of this sex-linked, X chromosome disorder are manifested almost entirely in males, although the gene for the disorder is inherited from the mother. Females have two X chromosomes while males have only one, lacking a 'back up' copy for the defective gene. In about 30% of cases of Hemopilia B, however, there is no family history of the disorder and the condition is the result of a spontaneous gene mutation[3]. A mother who is a carrier has a 50% chance of passing the faulty X chromosome to her daughter. That does not give the daughter the hemophilia disease, but does result in the daughter becoming a hemophilia carrier. Females are almost exclusively carriers of the disorder, and may have inherited it from either their mother or father. Sex-linked genes are those carried on the mammalian X chromosome but not the Y chromosome. ... ‹ The template below (Expand) is being considered for deletion. ...


These genetic deficiencies may lower blood plasma clotting factor levels of coagulation factors needed for a normal clotting process. When a blood vessel is injured, a temporary scab does form, but the missing coagulation factors prevent fibrin formation which is necessary to maintain the blood clot. Thus a haemophiliac does not bleed more intensely than a normal person, but for a much longer amount of time. In severe haemophiliacs even a minor injury could result in blood loss lasting days, weeks, or not ever healing completely. The critical risk here is with normally small injuries which, due to missing factor VIII, take long times to heal. In areas such as the brain or inside joints this can be fatal or life debilitating. Coagulation is the thickening or congealing of any liquid into solid clots. ...


The bleeding with external injury is normal, but incidence of late re-bleeding and internal bleeding is increased, especially into muscles, joints, or bleeding into closed spaces. Major complications include hemarthrosis, hemorrhage, gastrointestinal bleeding, and menorrhagia. To meet Wikipedias quality standards, this article or section may require cleanup. ... This article or section does not adequately cite its references or sources. ... Hemarthrosis (or haemarthrosis, plural h(a)emarthroses) is a bleeding into joint spaces. ... To meet Wikipedias quality standards, this article or section may require cleanup. ... Gastrointestinal bleeding describes every form of hemorrhage (blood loss) in the gastrointestinal tract, from the pharynx to the rectum. ... Menorrhagia is an abnormally heavy and prolonged menstrual period at regular intervals. ...

Contents

Causes

Hemophilia is nearly always caused by a genetic error causing the lack of a normally functioning clotting factor:

  • Hemophilia A involves a lack of functional clotting Factor VIII. (This represents 90% of haemophilia cases.)[4]
  • Hemophilia B involves a lack of functional clotting Factor IX.
  • Hemophilia C involves a lack of functional clotting Factor XI.
  • Hypofibrinogenemia involves a lack of functional clotting factor Factor I. Because it is so rare, about 1 to 2 cases per million births, it has no definite treatment approved by the FDA. It affects males and females equally. The blood of people with Hypofibrinogenemia neither clots nor contains sufficient amounts of Fibrinogen.

Haemophilia A (also spelt Hemophilia A or Hæmophilia A) is a blood clotting disorder caused by a mutation of the Factor VIII gene, leading to a deficiency in Factor VIII. It is the most common hemophilia. ... Factor VIII (FVIII) is an essential clotting factor. ... Haemophilia B (also spelled Hemophilia B or Hæmophilia B) is a blood clotting disorder caused by a mutation of the Factor IX gene. ... Factor IX (or Christmas factor or Christmas-Eve factor) is one of the serine proteases (EC 3. ... A mild form of hemophilia that mainly occurs in Jews of Ashkenazi descent. ... Factor XI or plasma thromboplastin antecent is one of the enzymes ( EC 3. ... Fibrin is a protein involved in the clotting of blood. ...

Occurrence

Hemophilia is quite rare only about 1 in every 10,000 births (or 1 in 5,000 male births) for hemophila A and 1 in 50,000 births for hemophila B.[5] About 18,000 people in the United States have hemophilia. Each year in the US, about 400 babies are born with the disorder. Hemophilia usually occurs in males and less often in females.[6] It is estimated that about 2500 Canadians have hemophilia A and about 500 Canadians have hemophila b. [7]


History

A young Queen Victoria passed hemophilia on to many of her descendants.
A young Queen Victoria passed hemophilia on to many of her descendants.

The earliest possible implicit reference to hemophilia may have been in the Talmud[8], a Jewish holy text, which states that males did not have to be circumcised if two brothers had already died from the procedure. In 1000, the Arab physician Abu al-Qasim al-Zahrawi (known as Albucasis in the West) wrote a more explicit description of hemophilia in his Al-Tasrif, in which he wrote of an Andalusian family whose males died of bleeding after minor injuries.[9] Image File history File links Size of this preview: 445 × 600 pixel Image in higher resolution (840 × 1132 pixel, file size: 81 KB, MIME type: image/jpeg) +/- File links The following pages on the English Wikipedia link to this file (pages on other projects are not listed): Victoria of the... Image File history File links Size of this preview: 445 × 600 pixel Image in higher resolution (840 × 1132 pixel, file size: 81 KB, MIME type: image/jpeg) +/- File links The following pages on the English Wikipedia link to this file (pages on other projects are not listed): Victoria of the... Victoria Queen of the United Kingdom of Great Britain and Ireland, Empress of India Victoria (Alexandrina Victoria) (24 May 1819–22 January 1901) was a Queen of the United Kingdom, reigning from 20 June 1837 until her death. ... The Talmud (Hebrew: ) is a record of rabbinic discussions pertaining to Jewish law, ethics, customs, and history. ... For other uses, see Jew (disambiguation). ... This article is being rewritten at Circumcision/temp Circumcision is the removal of some or all of the prepuce or foreskin though often the frenulum is also excised. ... Europe in 1000 The year 1000 of the Gregorian Calendar was the last year of the 10th century as well as the last year of the first millennium. ... In the history of medicine, Islamic medicine or Arabic medicine refers to medicine developed in the medieval Islamic civilisation and written in Arabic, the lingua franca of the Islamic civilization. ... Abu al-Qasim Khalaf ibn al-Abbas Al-Zahrawi (936 - 1013), (Arabic: أبو القاسم بن خلف بن العباس الزهراوي) also known in the West as Abulcasis, was an Andalusian-Arab physician, surgeon, and scientist. ... Al-Tasrif was an influential medieval treatise on medicine. ... Al-Andalus is the Arabic name given the Iberian Peninsula by its Muslim conquerors; it refers to both the Caliphate proper and the general period of Muslim rule (711–1492). ...


In 1803, Dr. John Conrad Otto, a Philadelphia physician, wrote an account about "a hemorrhagic disposition existing in certain families." He recognized that the disorder was hereditary and that it affected males and rarely females. He was able to trace the disease back to a woman who settled near Plymouth in 1720. The first usage of the term "hemophilia" appears in a description of the condition written by Hopff at the University of Zurich in 1828.[10] In 1937, Patek and Taylor, two doctors from Harvard, discovered anti-hemophilic globulin. Pavlosky, a doctor from Buenos Aires, found Hemophilia A and Hemophilia B to be separate diseases by doing a lab test. This test was done by transferring the blood of one hemophiliac to another hemophiliac. The fact that this corrected the clotting problem showed that there was more than one form of hemophilia. 1803 was a common year starting on Saturday (see link for calendar). ... // Events January 6 - The Committee of Inquiry on the South Sea Bubble publishes its findings February 11 - Sweden and Prussia sign the (2nd Treaty of Stockholm) declaring peace. ... Year 1828 (MDCCCXXVIII) was a leap year starting on Tuesday (link will display the full calendar) of the Gregorian Calendar (or a leap year starting on Thursday of the 12-day slower Julian calendar). ... Year 1937 (MCMXXXVII) was a common year starting on Friday (link will display the full calendar) of the Gregorian calendar. ... Harvard University is a private university in Cambridge, Massachusetts, USA, and a member of the Ivy League. ... Factor VII (formerly known as proconvertin) is one of the central proteins in the coagulation cascade. ... Haemophilia A (also spelt Hemophilia A or Hæmophilia A) is a blood clotting disorder caused by a mutation of the Factor VIII gene, leading to a deficiency in Factor VIII. It is the most common hemophilia. ... Haemophilia B (also spelled Hemophilia B or Hæmophilia B) is a blood clotting disorder caused by a mutation of the Factor IX gene. ...


Haemophilia in European royalty featured prominently and thus is sometimes known as "the royal disease". Queen Victoria passed the mutation to her son Leopold and, through several of her daughters, to various royals across the continent, including the royal families of Spain, Germany, and Russia. Tsarevich Alexei Nikolaevich, son of Nicholas II, was a descendant of Queen Victoria and suffered from hemophilia. It was claimed that Rasputin was successful at treating the Tsarevich Alexei of Russia's hemophilia. At the time, a common treatment administered by professional doctors was to use aspirin, which worsened rather than lessen the problem. It is believed that, by simply advising against the medical treatment, Rasputin could bring visible and significant improvement to the condition of Alexei. Haemophilia figured prominently in the history of European royalty. ... Queen Victoria redirects here. ... Prince Leopold, Duke of Albany (Leopold George Duncan Albert) (7 April 1853 – 28 March 1884), was a member of the British Royal Family, a son of Queen Victoria. ... The House of Hohenzollern is a German dynasty of electors, kings, and emperors of Prussia, Germany, and Romania. ... The House of Romanov (Рома́нов, pronounced ) was the second and last imperial dynasty of Russia, which ruled the country for five generations from 1613 to 1761. ... Tsarevich Alexei Nikolaevich Romanov (Russian: ), full title: Heir, Tsarevich and Grand Duke (Russian: ) (12 August [O.S. 30 July] 1904 — July 17, 1918), of the House of Romanov, was Tsarevich - the heir apparent - of Russia, being the youngest child and the only son of Tsar Nicholas II of Russia and... Nicholas II can refer to: Pope Nicholas II Tsar Nicholas II of Russia This is a disambiguation page — a navigational aid which lists other pages that might otherwise share the same title. ... Grigori Rasputin Grigori Yefimovich Rasputin (Russian: ) (22 January [O.S. 10 January] 1869 – 29 December [O.S. 16 December] 1916) was a Russian mystic with an influence in the later days of Russias Romanov dynasty. ... Tsarevich Alexei (1904-1918) Tsesarevich (Tsarevich) Alexei Nikolaevich of Russia (In Russian Царевич Алексей Николаевич) (August 12, 1904 - July 17, 1918), of the House of Romanov, was a Tsarevich of Russia and was the youngest child of Tsar Nicholas II of Russia and Alexandra of Hesse. ...


Prior to 1985, there were no laws enacted within the U.S. to screen blood. As a result, many hemophilia patients who received untested and unscreened clotting factor prior to 1992 were at an extreme risk for contracting HIV and Hepatitis C via these blood products. It is estimated that more than 50% of the Hemophilia population, over 10,000 people, contracted HIV from the tainted blood supply in the United States alone.[11] Species Human immunodeficiency virus 1 Human immunodeficiency virus 2 Human immunodeficiency virus (HIV) is a retrovirus that causes acquired immunodeficiency syndrome (AIDS, a condition in humans in which the immune system begins to fail, leading to life-threatening opportunistic infections). ... This page is for the disease. ...


As a direct result of the contamination of the blood supply in the late 1970s and early/mid 1980s with viruses such as Hepatitis and HIV, new methods were developed in the production of clotting factor products. The initial response was to heat-treat (pasteurize) plasma-derived factor concentrate, followed by the development of monoclonal factor concentrates, which use a combination of heat treatment and affinity chromatography to inactivate any viral agents in the pooled plasma from which the factor concentrate is derived. The Lindsay Tribunal in Ireland investigated, among other things, the slow adoption of the new methods. It is now well known that during the late 1970s and 1980s, large numbers of hemophiliacs became infected with HIV after receiving tainted clotting substances made by Armour Pharmaceutical Company, Bayer Corporation and its Cutter Biological division, Baxter International and its Hyland Pharmaceutical division and Alpha Therapeutic Corporation. ... Hepatitis (plural hepatitides) implies injury to liver characterised by presence of inflammatory cells in the liver tissue. ... Species Human immunodeficiency virus 1 Human immunodeficiency virus 2 Human immunodeficiency virus (HIV) is a retrovirus that causes acquired immunodeficiency syndrome (AIDS, a condition in humans in which the immune system begins to fail, leading to life-threatening opportunistic infections). ... Pasteurization is the process of heating food for the purpose of killing harmful organisms such as bacteria, viruses, protozoa, molds, and yeasts. ... The Lindsay Tribunal was set up in Ireland in 1999 to investigate the infection of haemophiliacs with HIV and Hepatitis C from contaminated blood products supplied by the Blood Transfusion Service Board. ...


Genetics

Females possess two X-chromosomes, whereas males have one X and one Y chromosome. Since the mutations causing the disease are recessive, a woman carrying the defect on one of her X-chromosomes may not be affected by it, as the equivalent allele on her other chromosome should express itself to produce the necessary clotting factors. However the Y-chromosome in men has no gene for factors VIII or IX. If the genes responsible for production of factor VIII or factor IX present on a male's X-chromosome are deficient there is no equivalent on the Y-chromosome, so the deficient gene is not masked by the dominant allele and he will develop the illness. X-linked Recessive inheritance From http://ghr. ... The human Y chromosome is one of two sex chromosomes, it contains the genes that cause testis development, thus determining maleness. ... It has been suggested that this article or section be merged into Dominance relationship. ... An allele (pronounced , ) (from the Greek αλληλος, meaning each other) is one member of a pair or series of different forms of a gene. ... For other uses, see Gene (disambiguation). ... Factor VIII (FVIII) is an essential clotting factor. ... Factor IX (or Christmas factor or Christmas-Eve factor) is one of the serine proteases (EC 3. ... An autosome is a non-sex chromosome. ...


Since a male receives his single X-chromosome from his mother, the son of a healthy female silently carrying the deficient gene will have a 50% chance of inheriting that gene from her and with it the disease; and if his mother is affected with haemophilia, he will have a 100% chance of being a haemophiliac. In contrast, for a female to inherit the disease, she must receive two deficient X-chromosomes, one from her mother and the other from her father (who must therefore be a haemophiliac himself). Hence haemophilia is far more common among males than females. However it is possible for female carriers to become mild haemophiliacs due to lyonisation (inactivation) of the X chromosomes. Haemophiliac daughters are more common than they once were, as improved treatments for the disease have allowed more haemophiliac males to survive to adulthood and become parents. Adult females may experience menorrhagia (heavy periods) due to the bleeding tendency. The pattern of inheritance is criss-cross type. This type of pattern is also seen in colour blindness. The Lyon hypothesis, conceived by Mary F. Lyon in 1961 while she was studying the genetic consequences of radiation exposure, states that in cells with multiple X chromosomes, all but one chromosome is inactivated during mammalian embryogenesis. ... Menorrhagia is an abnormally heavy and prolonged menstrual period at regular intervals. ... Color blindness in humans is the inability to perceive differences between some or all colors that other people can distinguish. ...


As with all genetic disorders, it is of course also possible for a human to acquire it spontaneously through mutation, rather than inheriting it, because of a new mutation in one of their parents' gametes. Spontaneous mutations account for about 33% of all haemophilia A and 20% of all hemophilia B cases. Genetic testing and genetic counseling is recommended for families with haemophilia. Prenatal testing, such as amniocentesis, is available to pregnant women who may be carriers of the condition. For linguistic mutation, see Apophony. ... Haemophilia A (also spelled Hemophilia A or Hæmophilia A) is a blood clotting disorder caused by a mutation of the factor VIII gene, leading to a deficiency in Factor VIII. It is the most common hemophilia. ... Haemophilia B (also spelled Hemophilia B or Hæmophilia B) is a blood clotting disorder caused by a mutation of the Factor IX gene. ... Genetic testing allows the genetic diagnosis of vulnerabilities to inherited diseases, and can also be used to determine a persons ancestry. ... Genetic counseling is the process by which patients or relatives, at risk of an inherited disorder, are advised of the consequences and nature of the disorder, the probability of developing or transmitting it, and the options open to them in management and family planning in order to prevent, avoid or... Prenatal diagnosis is the diagnosis of disease or condition in a fetus or embryo before it is born. ... Amniocentesis (also referred to as amniotic fluid test or AFT), is a medical procedure used in prenatal diagnosis of genetic risk factors, in which a small amount of amniotic fluid, which contains fetal tissues, is extracted from the amnion or amniotic sac surrounding a developing fetus, and the fetal DNA...


Probability

If a female gives birth to a haemophiliac child, either the female is a carrier for the disease or the haemophilia was the result of a spontaneous mutation. Until modern direct DNA testing, however, it was impossible to determine if a female with only healthy children was a carrier or not. Generally, the more healthy sons she bore, the higher the probability that she was not a carrier. If the RH factor of the born male is different from the mother, the child will not be affected. For linguistic mutation, see Apophony. ... Genetic fingerprinting, DNA testing, DNA typing, and DNA profiling are techniques used to distinguish between individuals of the same species using only samples of their DNA. Its invention by Dr. Alec Jeffreys at the University of Leicester was announced in 1985. ... The term Rhesus blood group system refers to the five main Rhesus antigens (C, c, D, E and e) as well as the many other less frequent Rhesus antigens. ...


If a male is afflicted with the disease and has children, his daughters will be carriers of haemophilia. His sons, however, will not be affected with the disease. This is because the disease is X-linked and the father cannot pass haemophilia through the Y chromosome. Males with the disorder are then no more likely to pass on the gene to their children than carrier females, though all daughter they sire will be carriers and all sons they father will not have hemophilia (unless the mother is a carrier).


Treatment

Though there is no cure for hemophilia, it can be controlled with regular infusions of the deficient clotting factor, i.e. factor VIII in haemophilia A or factor IX in hemophilia B. Factor replacement can be either isolated from human blood serum, recombinant, or a combination of the two. Some hemophiliacs develop antibodies (inhibitors) against the replacement factors given to them, so the amount of the factor has to be increased or non-human replacement products must be given, such as porcine factor VIII. Factor VIII (FVIII) is an essential clotting factor. ... Factor IX (or Christmas factor or Christmas-Eve factor) is one of the serine proteases (EC 3. ... Blood plasma is a component of blood. ... Recombinant proteins are proteins that are produced by different genetically modified organisms following insertion of the relevant DNA into their genome. ... Wikipedia does not yet have an article with this exact name. ... Species Sus barbatus Sus bucculentus Sus cebifrons Sus celebensis Sus domesticus Sus heureni Sus philippensis Sus salvanius Sus scrofa Sus timoriensis Sus verrucosus Pigs are ungulates native to Eurasia collectively grouped under the genus Sus within the Suidae family. ...


If a patient becomes refractory to replacement coagulation factor as a result of circulating inhibitors, this may be partially overcome with recombinant human factor VII (NovoSeven®), which is registered for this indication in many countries. Factor VII (formerly known as proconvertin) is one of the central proteins in the coagulation cascade. ...


In early 2008, the US Food and Drug Administration approved Xyntha® (Wyeth) anti-hemophilic factor, genetically engineered from the genes of Chinese hamster ovary cells. Since 1993 (Dr. Mary Nugent) recombinant factor products (which are typically cultured in Chinese hamster ovary (CHO) tissue culture cells and involve little, if any human plasma products) have been available and have been widely used in wealthier western countries. While recombinant clotting factor products offer higher purity and safety, they are, like concentrate, extremely expensive, and not generally available in the developing world. In many cases, factor products of any sort are difficult to obtain in developing countries. “FDA” redirects here. ... Wyeth, formerly known as American Home Products, is one of the largest pharmaceutical companies in the world. ... Chinese Hamster Ovary cells (CHO cells) are a cell line derived from Chinese Hamster ovary cells. ... Blood plasma is the liquid component of blood, in which the blood cells are suspended. ...


In Western countries, common standards of care fall into one of two categories: prophylaxis or on-demand. Prophylaxis involves the infusion of clotting factor on a regular schedule in order to keep clotting levels sufficiently high to prevent spontaneous bleeding episodes. On-demand treatment involves treating bleeding episodes once they arise. In 2007, a clinical trial was published in the New England Journal of Medicine (NEJM) comparing on-demand treatment of boys (< 30 months) with Hemophilia A with prophylactic treatment (infusions of 25 IU/kg body weight of Factor VIII every other day) in respect to its effect on the prevention of joint-diseases. When the boys reached 6 years of age, 93% of those in the prophylaxis group and 55% of those in the episodic-therapy group had a normal index joint-structure on MRI. [12] Prophylactic treatment, however, resulted in average costs of $300,000 per year. The author of an editorial published in the same issue of the NEJM demands more clinical studies addressing the cost-effectiveness of prophylactic treatment. [13] The New England Journal of Medicine (New Engl J Med or NEJM) is a peer-reviewed medical journal published by the Massachusetts Medical Society. ... Factor VIII (FVIII) is an essential clotting factor. ... The mri are a fictional alien species in the Faded Sun Trilogy of C.J. Cherryh. ... Health economics is a branch of economics concerned with issues related to scarcity in the allocation of health and health care. ... USD redirects here. ...


It is recommended that people affected with Hemophilia do specific exercises to strengthen the joints, particularly the elbows, knees, and ankles. Exercises include elements which increase flexibility, tone, and strength of muscles, increasing their ability to protect joints from damaging bleeds. These exercises are recommended after an internal bleed occurs and on a daily basis to strengthen the muscles and joints to prevent new bleeding problems. Many recommended exercises include standard sports warm-up and training exercises such as stretching of the calves, ankle circles, elbow flexions, and Quadriceps sets.


Alternative and complimentary treatments

Scientific studies indicate that hypnosis and self-hypnosis can be effective at reducing bleeds and the severity of bleeds and thus the frequency of factor treatment.[14] Herbs which strengthen blood vessels and act as astringents may also benefit patients with hemophilia. These herbs include: Bilberry(Vaccinium myrtillus), Grape seed extract (Vitis vinifera), Scotch broom (Cytisus scoparius), Stinging nettle (Urtica dioica), Witch hazel (Hamamelis virginiana), and yarrow (Achillea millefolium).[14] Binomial name Vaccinium myrtillus L. Bilberry is a name given to several species of low-growing shrubs in the genus Vaccinium (family Ericaceae) that bear tasty fruits. ... Binomial name Vitis vinifera L. For thousands of years, the fruit and plant of Vitis vinifera, the European grapevine, have been harvested for both medicinal and nutritional value; its history is intimately entwined with the history of wine. ... Binomial name Cytisus scoparius (L.) Link Synonyms Spartium scoparium L. Genista scoparius L. (Lam. ... Binomial name Urtica dioica L. The stinging nettle (Urtica dioica) is a herbaceous flowering plant native to Europe, Asia, northern Africa, and North America, and is the best known member of the nettle genus Urtica. ... Binomial name L. Hamamelis virginiana is a species of Witch-hazel found across eastern North America, from Nova Scotia to Wisconsin in the north and northern Florida to Texas in the south. ... Yarrow redirects here. ...


Differential diagnosis

Haemophilia A can be mimicked by von Willebrand Disease Von Willebrand disease (vWD) is the most common hereditary coagulation abnormality described in humans, although it can also be acquired as a result of other medical conditions. ...

  • von Willebrand Disease type 2A, where decreased levels of von Willebrand Factor can lead to premature proteolysis of Factor VIII. In contrast to haemophilia, vWD type 2A is inherited in an autosomal dominant fashion.
  • von Willebrand Disease type 2N, where von Willebrand Factor cannot bind Factor VIII, autosomal recessive inheritance. (ie; both parents need to give the child a copy of the gene). [1]
  • von Willebrand Disease type 3, where lack of von Willebrand Factor causes premature proteolysis of Factor VIII. In contrast to haemophilia, vWD type 3 is inherited in an autosomal recessive fashion.

In genetics, dominance describes a specific relationship between the effects of different versions of a gene (alleles) on a trait (phenotype). ...

References

  1. ^ Douglas Harper. Online Etymology Dictionary.
  2. ^ Hemophilia B. Retrieved on 2007-11-21.
  3. ^ Hemophilia B. Retrieved on 2007-11-21.
  4. ^ www.wrongdianosis.com/h/hemophilia/stats.htm#medical_stats
  5. ^ World Federation of Hemophila Frequently Asked Questions About Hemophilia
  6. ^ U.S. National Library of Medicine. Retrieved on 2007-12-02.
  7. ^ Canadian Hemophila Society FAQ
  8. ^ Tractate Yevamot 64b
  9. ^ Patricia Skinner (2001), Unani-tibbi, Encyclopedia of Alternative Medicine
  10. ^ The History of hemophilia. Retrieved on 2007-06-27.
  11. ^ In re Rhone-Poulenc Rorer Inc., 51 F.3d 1293, 1296 (7th Cir. 1995), http://www.projectposner.org/case/1995/51F3d1293 (PDF courtesy link). Retrieved 2008-01-28
  12. ^ Manco-Johnson MJ, Abshire TC, Shapiro AD, Riske B, Hacker MR, Kilcoyne R, Ingram JD, Manco-Johnson ML, Funk S, Jacobson L, Valentino LA, Hoots WK, Buchanan GR, DiMichele D, Recht M, Brown D, Leissinger C, Bleak S, Cohen A, Mathew P, Matsunaga A, Medeiros D, Nugent D, Thomas GA, Thompson AA, McRedmond K, Soucie JM, Austin H, Evatt BL. Prophylaxis versus episodic treatment to prevent joint disease in boys with severe haemophilia. N Engl J Med. 2007 Aug 9;357(6):535-44. PMID 17687129
  13. ^ Roosendaal G, Lafeber F. Prophylactic treatment for prevention of joint disease in hemophilia--cost versus benefit. N Engl J Med. 2007 Aug 9;357(6):603-5. PMID 17687136
  14. ^ a b University of Maryland Medical Center Complimentary medicines: Hemophilia

Year 2007 (MMVII) is the current year, a common year starting on Monday of the Gregorian calendar and the AD/CE era in the 21st century. ... is the 325th day of the year (326th in leap years) in the Gregorian calendar. ... Year 2007 (MMVII) is the current year, a common year starting on Monday of the Gregorian calendar and the AD/CE era in the 21st century. ... is the 325th day of the year (326th in leap years) in the Gregorian calendar. ... Year 2007 (MMVII) is the current year, a common year starting on Monday of the Gregorian calendar and the AD/CE era in the 21st century. ... is the 336th day of the year (337th in leap years) in the Gregorian calendar. ... Year 2007 (MMVII) is the current year, a common year starting on Monday of the Gregorian calendar and the AD/CE era in the 21st century. ... is the 178th day of the year (179th in leap years) in the Gregorian calendar. ... 2008 (MMVIII) is the current year, a leap year that started on Tuesday of the Anno Domini (or common era), in accordance to the Gregorian calendar. ... is the 28th day of the year in the Gregorian calendar. ... Image File history File links Question_book-3. ...

External links

Leukopenia or leukocytopenia refers to a decrease in the number of circulating white blood cells (leukocytes) in the blood. ... Eosinophil granulocyte Basophil granulocyte Granulocytes are a category of white blood cells characterised by the presence of granules in their cytoplasm. ... Myeloid cells is a subsummating term for all hemopoietic cells except the lymphoid ones (T-cells, B-cells, NK-cells, dendritic cells). ... Leukocytosis is an elevation of the white blood cell count (the leukocyte count) above the normal range. ... In medicine, granulocytosis is the presence in peripheral blood of an increased number of granulocytes, a category of white blood cells. ... Neutrophilia (or neutrophil leukocytosis) is a condition where a person has a high number of neutrophil granulocytes in their blood. ... Eosinophilia is the state of having high eosinophil granulocytes in the blood. ... Leukopenia or leukocytopenia refers to a decrease in the number of circulating white blood cells (leukocytes) in the blood. ... Agranulocytosis (literally meaning an increase of agranulocytes or more commonly, a lack of granulocytes) is an acute condition involving a severe and dangerous leukopenia (reduction in the number of white blood cells) in the body. ... Neutropenia (or neutropaenia, adjective neutrop(a)enic) is a hematological disorder characterized by an abnormally low number of neutrophil granulocytes (a type of white blood cell). ... Kostmann syndrome is a rare inherited form of neutropenia usually detected soon after birth. ... Myeloid cells is a subsummating term for all hemopoietic cells except the lymphoid ones (T-cells, B-cells, NK-cells, dendritic cells). ... Chronic granulomatous disease (CGD) is a diverse group of hereditary diseases in which certain cells of the immune system have difficulty forming the reactive oxygen compounds (most importantly, the superoxide radical) used to kill certain ingested pathogens. ... A scanning electron microscope (SEM) image of a single human lymphocyte. ... Leukocytosis is an elevation of the white blood cell count (the leukocyte count) above the normal range. ... Lymphocytosis is an increase in the number of lymphocytes in the blood. ... Leukopenia or leukocytopenia refers to a decrease in the number of circulating white blood cells (leukocytes) in the blood. ... Lymphopenia is the condition in which there exists an abnormally low number of lymphocytes in the blood. ... Asplenia refers to the absence (a-) of normal spleen function and is associated with some risks. ... Methemoglobinemia, also known as met-Hb, is a disorder characterized by the presence of a higher than normal level of methemoglobin in the blood. ... Pancytopenia is a medical condition in which there is a reduction in the number of red and white blood cells, as well as platelets. ...

  Results from FactBites:
 
Haemophilia (545 words)
Haemophilia is a rare blood disorder caused by an inherited gene.
Haemophilia is a blood clotting disorder in which one of the essential clotting factors is deficient.
When the mother carries the haemophilia gene and the father is unaffected there will be a 50 per cent chance at each birth that their daughter may carry the haemophilia gene or that their son may have haemophilia.
NNHF - About Haemophilia (294 words)
Haemophilia is a congenital bleeding disorder that affects males.
Those with haemophilia B have similar problems with Factor IX (FIX).  Haemophilia is characterised as "severe" when the activity of the affected clotting factor (FVIII or FIX) is less than 1% of normal.
Haemophilia is termed "mild" when the relevant clotting factor activity is greater than 5%, but less than normal and "moderate" when clotting factor activity is between 1% and 5% of normal.  Approximately 50% of haemophilia patients have severe disease and can require treatment for bleeding several times per month.
  More results at FactBites »

 
 

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